Autoimmune hemolytic anemia as a complication of congenital anemias. A case series and review of the literature

Irene Motta, Juri Giannotta, Marta Ferraresi, Kordelia Barbullushi, Nicoletta Revelli, Giovanna Graziadei, Wilma Barcellini, Bruno Fattizzo

Research output: Contribution to journalArticlepeer-review


Congenital anemias may be complicated by immune-mediated hemolytic crisis. Alloantibodies are usually seen in chronically transfused patients, and autoantibodies have also been described, although they are rarely associated with overt autoimmune hemolytic anemia (AIHA), a serious and potentially life-threatening complication. Given the lack of data on the AIHA diagnosis and management in congenital anemias, we retrospectively evaluated all clinically relevant AIHA cases occurring at a referral center for AIHA, hemoglobinopathies, and chronic hemolytic anemias, focusing on clinical management and outcome. In our cohort, AIHA had a prevalence of 1% (14/1410 patients). The majority were warm AIHA. Possible triggers were recent transfusion, infection, pregnancy, and surgery. All the patients received steroid therapy as the first line, and about 25% required further treatment, including rituximab, azathioprine, intravenous immunoglobulins, and cyclophosphamide. Transfusion support was required in 57% of the patients with non-transfusion-dependent anemia, and recombinant human erythropoietin was safely administered in one third of the patients. AIHA in congenital anemias may be challenging both from a diagnostic and a therapeutic point of view. A proper evaluation of hemolytic markers, bone marrow compensation, and assessment of the direct antiglobulin test is mandatory.

Original languageEnglish
Article number3439
JournalJournal of Clinical Medicine
Issue number15
Publication statusPublished - Aug 1 2021


  • Alloimmunization
  • Autoimmune hemolytic anemia
  • Congenital hemolytic anemias
  • Sickle cell disease
  • Thalassemia

ASJC Scopus subject areas

  • Medicine(all)


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